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Vasculitides Including Henoch–Schönlein Purpura

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Textbook of Pediatric Gastroenterology, Hepatology and Nutrition

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Abstract

Inflammation of the blood vessels (vasculitis) is uncommon in childhood with the exception of Henoch–Schönlein purpura and Kawasaki’s disease. The disease can be classified according to the size of blood vessel affected. The gastrointestinal (GI) clinical manifestations vary according to the size and type of blood vessel involved but include GI bleeding, gastrointestinal ulceration, GI obstruction, GI perforation and abdominal pain. Nonspecific mucosal inflammation might also be present. Diagnosis is often delayed because the presentation is so varied. Diagnostic evaluation may include GI panendoscopy, videocapsule endoscopy, abdominal ultrasound and/or MRI and visceral angiography. Treatment is disease and presentation specific.

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References

  1. Morgan MD, Savage COS. Vasculitis in the gastrointestinal tract. Best Pract Res Clin Gastroenterol. 2005;19(2):215–33.

    Article  PubMed  Google Scholar 

  2. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 27 Dec 2012;65(1):1–11. doi:10.1002/art.37715.

    Google Scholar 

  3. Peru H, Soylemezoglu O, Bakkaloglu SA, Elmas S, Bozkaya D, Elmaci AM, et al. Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period. Clin Rheumatol. 2008;27(9):1087–92.

    Article  PubMed  Google Scholar 

  4. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69(5):798–806.

    Article  PubMed  Google Scholar 

  5. Chang W-L, Yang Y-H, Lin Y-T, Chiang B-L. Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients. Acta Paediatr. 2004;93(11):1427–31.

    Article  PubMed  Google Scholar 

  6. Nchimi A, Khamis J, Paquot I, Bury F, Magotteaux P. Significance of bowel wall abnormalities at ultrasound in Henoch-Schönlein purpura. J Pediatr Gastroenterol Nutr. 2008;46(1):48–53.

    Article  PubMed  Google Scholar 

  7. Hsu H-L, Hsiao C-H, Liu K-L. Henoch-Schönlein purpura. Clin Gastroenterol Hepatol. 2010;8(8):e83–4.

    Article  PubMed  Google Scholar 

  8. Falcini F, Cimaz R, Calabri GB, Picco P, Martini G, Marazzi MG, et al. Kawasaki’s disease in northern Italy: a multicenter retrospective study of 250 patients. Clin Exp Rheumatol. 2002;20(3):421–6.

    CAS  PubMed  Google Scholar 

  9. Zulian F, Falcini F, Zancan L, Martini G, Secchieri S, Luzzatto C, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr. 2003;142(6):731–5.

    Article  PubMed  Google Scholar 

  10. Maurer K, Unsinn KM, Waltner-Romen M, Geiger R, Gassner I. Segmental bowel-wall thickening on abdominal ultrasonography: an additional diagnostic sign in Kawasaki disease. Pediatr Radiol. 2008;38(9):1013–6.

    Article  PubMed  Google Scholar 

  11. Eleftheriou D, Levin M, Shingadia D, Tulloh R, Klein N, Brogan P. Management of Kawasaki disease. Arch Dis Child. 2014;99(1):74–83.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Eleftheriou D, Dillon MJ, Tullus K, Marks SD, Pilkington CA, Roebuck DJ, et al. Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years. Arthritis Rheum. 2013;65(9):2476–85.

    Article  CAS  PubMed  Google Scholar 

  13. Dulic-Lakovic E, Dulic M, Hubner D, Fuchssteiner H, Pachofszky T, Stadler B, et al. Bleeding Dieulafoy lesions of the small bowel: a systematic study on the epidemiology and efficacy of enteroscopic treatment. Gastrointest Endosc. 2011;74(3):573–80.

    Article  PubMed  Google Scholar 

  14. Maeda K, Hayashi Y, Morita I, Matsuoka O, Nishiyama M, Nishimura H, et al. Recurrent Dieulafoy’s ulcers in the stomach and colonic perforation caused by polyarteritis nodosa: report of a case. Gastrointest Endosc. 2006;63(2):349–52.

    Article  PubMed  Google Scholar 

  15. Vavricka SR, Dirnhofer S, Degen L. Polyarteritis nodosa mimicking inflammatory bowel disease. Clin Gastroenterol Hepatol. 2007;5(11):A22.

    Article  PubMed  Google Scholar 

  16. Gündoğdu HZ, Kale G, Tanyel FC, Büyükpamukçu N, Hiçsönmez A. Intestinal perforation as an initial presentation of polyarteritis nodosa in an 8-year-old boy. J Pediatr Surg. 1993;28(4):632–4.

    Article  PubMed  Google Scholar 

  17. Venuta A, Ceccarelli PL, Biondini D, Montanari F. Jejunal obstruction as initial presentation of polyarteritis nodosa in a 13-month-old boy. J Pediatr Surg. 2011;46(7):E27–9.

    Article  PubMed  Google Scholar 

  18. Brogan PA, Malik M, Shah N, Kilday JP, Ramsay A, Shah V, et al. Systemic vasculitis: a cause of indeterminate intestinal inflammation. J Pediatr Gastroenterol Nutr. 2006;42(4):405–15.

    Article  CAS  PubMed  Google Scholar 

  19. Koné-Paut I, Darce-Bello M, Shahram F, Gattorno M, Cimaz R, Ozen S, et al. Registries in rheumatological and musculoskeletal conditions. Pediatric Behçet’s disease: an international cohort study of 110 patients. One-year follow-up data. Rheumatology (Oxford). 2011;50(1):184–8.

    Article  Google Scholar 

  20. Mora P, Menozzi C, Orsoni JG, Rubino P, Ruffini L, Carta A. Neuro-Behçet’s disease in childhood: a focus on the neuro-ophthalmological features. Orphanet J Rare Dis. 2013;8:18.

    Article  PubMed Central  PubMed  Google Scholar 

  21. Zwerina J, Eger G, Englbrecht M, Manger B, Schett G. Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum. 2009;39(2):108–15.

    Article  PubMed  Google Scholar 

  22. Villiger PM, Guillevin L. Microscopic polyangiitis: clinical presentation. Autoimmun Rev. 2010;9(12):812–9.

    Article  PubMed  Google Scholar 

  23. Brogan P, Eleftheriou D, Dillon M. Small vessel vasculitis. Pediatr Nephrol. 2010;25(6):1025–35.

    Article  PubMed Central  PubMed  Google Scholar 

  24. Cakar N, Yalcinkaya F, Duzova A, Caliskan S, Sirin A, Oner A, et al. Takayasu arteritis in children. J Rheumatol. 2008;35(5):913–9.

    PubMed  Google Scholar 

  25. Eleftheriou D, Brogan PA. Vasculitis in children. Best Pract Res Clin Rheumatol. 2009;23(3):309–23.

    Article  PubMed  Google Scholar 

  26. Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med. 28 Feb 2008;358(9):929–39.

    Article  CAS  PubMed  Google Scholar 

  27. Tu Y-L, Yeh K-W, Chen L-C, Yao T-C, Ou L-S, Lee W-I, et al. Differences in disease features between childhood-onset and adult-onset systemic lupus erythematosus patients presenting with acute abdominal pain. Semin Arthritis Rheum. 2011;40(5):447–54.

    Article  PubMed  Google Scholar 

  28. Lee CK, Lee TH, Lee S-H, Chung I-K, Park S-H, Kim H-S, et al. GI vasculitis associated with systemic lupus erythematosus. Gastrointest Endosc. 2010;72(3):618–9.

    Article  PubMed  Google Scholar 

  29. Dillon MJ. Childhood vasculitis. Lupus. 1998;7(4):259–65.

    Article  CAS  PubMed  Google Scholar 

  30. Lee SY, Lee SW, Chung WT. Jejunal vasculitis in patient with rheumatoid arthritis: case report and literature review. Mod Rheumatol. 2012;22(6):924–7.

    Article  PubMed  Google Scholar 

  31. Lee SK, Kim BK, Kim TI, Kim WH. Differential diagnosis of intestinal Behçet“s disease and Crohn”s disease by colonoscopic findings. Endoscopy. 2009;41(1):9–16.

    Article  CAS  PubMed  Google Scholar 

  32. Hokama A, Kishimoto K, Ihama Y, Kobashigawa C, Nakamoto M, Hirata T, et al. Endoscopic and radiographic features of gastrointestinal involvement in vasculitis. World J Gastrointest Endosc. 16 March 2012;4(3):50–6.

    Article  PubMed Central  PubMed  Google Scholar 

  33. Preud’Homme DL, Michail S, Hodges C, Milliken T, Mezoff AG. Use of wireless capsule endoscopy in the management of severe Henoch-Schonlein purpura. Pediatrics. 2006;118(3):e904–6.

    Article  PubMed  Google Scholar 

  34. Bua J, Lepore L, Martelossi S, Ventura A. Video capsule endoscopy and intestinal involvement in systemic vasculitis. Dig Liver Dis. 2008;40(11):905.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Division of Neurogastroenterology and Motility, Department of Gastroenterology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, WC1N 3JH, London, UK

    Keith J. Lindley

  2. Division of Intestinal Rehabilitation and Nutrition, Department of Gastroenterology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

    Jutta Köglmeier

Authors
  1. Keith J. Lindley
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  2. Jutta Köglmeier
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Corresponding author

Correspondence to Keith J. Lindley .

Editor information

Editors and Affiliations

  1. Department of Pediatrics, University of Chicago, Chicago, Illinois, USA

    Stefano Guandalini

  2. Clinical Director Child Health King’s College Hospital, Professor and Director Pediatric Liver, GI and Nutrition Center, London, United Kingdom

    Anil Dhawan

  3. Department of Pediatrics, Hebrew University Hadassah Hospital, Jerusalem, Israel

    David Branski

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Lindley, K., Köglmeier, J. (2016). Vasculitides Including Henoch–Schönlein Purpura. In: Guandalini, S., Dhawan, A., Branski, D. (eds) Textbook of Pediatric Gastroenterology, Hepatology and Nutrition. Springer, Cham. https://doi.org/10.1007/978-3-319-17169-2_31

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  • DOI: https://doi.org/10.1007/978-3-319-17169-2_31

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17168-5

  • Online ISBN: 978-3-319-17169-2

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